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Interleukin 10 (IL-10) plays a role in inflammation and cell-type responses. The anti-SS-A/Ro antibody contributes to leucopenia, and cutaneous and neonatal lupus. Objectives: To evaluate the association between serum IL-10 levels and autoantibodies, disease activity and organ involvement in systemic lupus erythematosus (SLE) patients. Patients and methods: We studied 200 SLE patients and 50 controls. We analyzed organ involvement, disease activity, serum IL-10 and interleukin-6 (IL-6) levels, and antinuclear and antiphospholipid antibody profiles. Results: Serum IL-10 and IL-6 levels were higher in SLE patients than in controls (all p < 0.00001). Serum IL-10 levels were positively correlated with IL-6 (p < 0.00001), CRP (p < 0.00001), fibrinogen (p = 0.003), and ESR (p < 0.00001), and negatively correlated with hemoglobin (p = 0.0004) and lymphocytes (p = 0.01). Serum IL-6 levels were positively correlated with CRP (p < 0.00001), fibrinogen (p = 0.001), and ESR (p < 0.00001); and negatively correlated with hemoglobin (p = 0.008) and lymphocytes (p = 0.03). Elevated serum IL-10 levels were associated with an increased risk of anti-SS-A/Ro antibody positivity (p = 0.03). Elevated serum IL-6 levels were associated with an increased risk of heart (p = 0.007) and lung (p = 0.04) involvement. Conclusions: In SLE patients, increased serum IL-10 levels were associated with increased disease activity and risk of anti–SS-A/Ro antibody positivity.

OBJECTIVES: The study aimed to characterise the Polish population of (ANCA)-associated vasculitides (AAV) with respiratory involvement (RI), in comparison to the subgroup without lung manifestations and the other cohorts. METHODS: Retrospective analysis of the Polish population of AAV with RI was conducted, based on data from the POLVAS registry. Standard descriptive statistics, χ2 test, and Mann-Whitney U test were used to perform comparisons. RESULTS: Among 461 cases qualified to this study, there were 316 cases with RI (68.5%), 206 with granulomatosis with polyangiitis (GPA) (65.2%), 80 with eosinophilic granulomatosis with polyangiitis (EGPA) (25.3%) and 30 with microscopic polyangiitis (MPA) (9.5%). Proportion of RI in GPA, MPA, and EGPA accounted for 67.8%; 40.0%; 97.6%, respectively. The number of relapses was higher in the RI group (median 1.0 vs. 0.0; p=0.01). In the subgroup of combined GPA and MPA with RI, the trends toward higher proportion of deaths (11.7% vs. 5.7%; p=0.07), relapses requiring hospitalisation (52.2% vs. 42.4%, p=0.07) and relapses requiring admission to the intensive care unit (5.6% vs. 1.4%, p=0.09) were observed, median maximal concentration of CRP was higher (46 vs. 25 mg/l; p=0.01) and more aggressive treatment was administered. CONCLUSIONS: Prevalence of RI in the Polish population of AAV is similar to the values reported in the literature, however, the proportion observed in GPA is closer to those presented in Asian than Western European cohorts. RI seems to be associated with a more severe course of disease and its presence prompts more aggressive treatment.

Objective We aimed to assess patients with axial spondyloarthritis (axSpA) and inflammatory bowel disease (IBD) for disease activity and serum markers of endothelial dysfunction. Methods We studied 161 patients (123 males, 38 females) with axSpA: 153 with ankylosing spondylitis and 8 with non-radiographic axSpA, and 30 healthy controls (HC). We collected: age; sex; disease duration; extra-articular symptoms (IBD and acute anterior uveitis), comorbidities; human leukocyte antigen B27 status; and treatment. We measured serum interleukin (IL)-6, interleukin-18, IL-23, vascular endothelial growth factor (VEGF) epidermal growth factor (EGF), asymmetric dimethylarginine (ADMA), endothelin-1 (ET-1), and fetuin-A levels. Results IBD was diagnosed in 19 (11.8%) patients with axSpA. Compared to patients with axSpA without IBD, those with IBD had higher serum C-reactive protein (CRP) level (p = 0.05), erythrocyte sedimentation rate (ESR) (p = 0.005), and serum ET-1 levels (p = 0.01). In patients with axSpA and IBD, ET-1 levels correlated positively with CRP level (p = 0.006) and ESR (p = 0.02), and ADMA levels with visual analog scale scores (p = 0.01). Patients with axSpA and IBD had higher serum levels of IL-6 (p = 0.01), IL-18 (p = 0.005), and ADMA (p = 0.01) and lower serum levels of fetuin-A (p = 0.01) than did controls. Conclusions Patients with axSpA and IBD had higher levels of disease activity, as assessed by ESR and CRP and ET-1 levels, than did patients with axSpA without IBD. Compared to HC, patients with axSpA and IBD had increased IL-18, ADMA levels and decreased fetuin-A level.

Aktualna strategia leczenia systemowego w zesztywniającym zapaleniu stawow kregoslupa (ZZSK) obejmuje niesteroidowe leki przeciwzapalne, inhibitory TNF-alfa (iTNF) oraz inhibitory IL-17. Inhibitory TNF stanowią bardzo skuteczną opcje terapeutyczną u pacjentow z osiową spondyloartropatią, ale cześc chorych nie odpowiada na stosowane leczenie lub wystepują u nich objawy niepoządane. Sekukinumab jest w pelni ludzkim przeciwcialem monoklonalnym przeciwko IL-17A, ktorego skutecznośc w leczeniu pacjentow z ZZSK potwierdzono w badaniach klinicznych. Sekukinumab szybko i trwale zmniejsza aktywnośc choroby w stopniu porownywalnym z inhibitorami TNF. Prowadzi takze do istotnej poprawy jakości zycia oraz zmniejsza stopien niesprawności i zmeczenia u pacjentow z aktywnym ZZSK. Sekukinumab jest zarejestrowany do leczenia pacjentow z ZZSK i luszczycowym zapaleniem stawow (ŁZS) od 2015 roku. Oprocz korzystnego efektu klinicznego sekukinumab hamuje postep uszkodzenia radiologicznego u okolo 80% pacjentow z ZZSK i okolo 84% pacjentow z ŁZS. Od 2020 roku sekukinumab jest zarejestrowany takze do leczenia pacjentow z osiową spondyloartropatią bez zmian radiograficznych (nr-axSpA). Wyniki rejestracyjnego badania klinicznego wykazaly skutecznośc sekukinumabu w tej grupie chorych w odniesieniu do wielu punktow koncowych, takich jak: ASAS40, BASDAI, BASFI, SF-36, ASQoL czy ASDAS-CRP. Odsetek pacjentow z nieaktywną chorobą wedlug ASDAS-CRP byl 2,5-krotnie wiekszy w grupie pacjentow leczonych sekukinumabem w porownaniu z placebo (RR = 2,55 [95% CI: 1,45; 4,47] p = 0,001). Dodatkowo u pacjentow leczonych sekukinumabem obserwowano zahamowanie objawow zapalenia w stawach krzyzowobiodrowych w badaniu rezonansu magnetycznego. Powyzsze wyniki pokazują, ze pacjenci z nr-axSpA zyskali nową skuteczną i bezpieczną opcje terapeutyczną. Biorąc pod uwage wysoką skutecznośc sekukinumabu w leczeniu pacjentow z ŁZS, w tym takich objawow, jak zapalenie stawow obwodowych, zapalenie ściegien i zapalenie palcow oraz luszczycy skory i paznokci, sekukinumab moze stanowic takze skuteczną opcje leczenia u cześci pacjentow z obwodową SpA.

IntroductionThe aim of the study was to evaluate the impact of disease activity, selected serum cytokines, and therapy on metabolic syndrome (MetS) components in patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.Material and methodsWe studied 46 SAPHO patients (40 women, 6 men). We recorded age, sex, disease duration, arthritis localization, type of skin changes, bone scintigraphy results, comorbidities, BASDAI, VAS, and treatment. We measured erythrocyte sedimentation rate, C-reactive protein, lipid profile, serum IL-6, IL-18, IL-23, endothelin-1, vascular endothelial growth factor, and epidermal growth factor (EGF).Results97.8% of patients had sternoclavicular joint arthritis, 91.3% of patients palmoplantar pustulosis. In 65.2% of SAPHO patients skin changes and arthritis started simultaneously. Apart from non-steroidal anti-inflammatory drugs, patients were treated with methotrexate (41.3%), sulfasalazine (41.3%), and antibiotics (39.1%). 19.5% of patients met MetS criteria. Serum IL-23 correlated positively with total cholesterol (TC; p = 0.02) and high-density lipoprotein cholesterol (HDL-C) (p = 0.01) in the SAPHO group. There was a negative correlation between HDL-C and BASDAI (p = 0.02). Patients treated with methotrexate had higher triglyceride (p = 0.01) and low-density lipoprotein cholesterol (LDL-C) (p = 0.01) levels. There was a negative correlation between TC and EGF (p = 0.03). Increased prevalence of autoimmune diseases and depression was observed in SAPHO patients.ConclusionsSerum IL-23 protects, whereas methotrexate treatment stimulates selected components of the MetS in patients with SAPHO syndrome.

Achieving remission or lowdisease activity (LDA) is an integral principle of treat‑to‑target (T2T) strategy in rheumatoid arthritis (RA). Prior studies have reported that achieving T2T therapeutic goals may be realistic only for a fraction of patients. Prospective, real‑world data on achieving target disease control in ambulatory care populations are limited for Central and Eastern European countries.The aim of the study was to analyze the efficacy of treatment and determine simple predictors of achieving T2T therapy goals in daily RA practice.This multicenter, 6‑month study evaluated therapy outcomes and clinical characteristics of 791 consecutive RA outpatients, meeting the preset criteria of inadequate disease control.Only 9% of RA patients achieved remission or LAD after 3 months and 35% after 6 months. Achieving treatment targets after 6 months was associated with lower rates of pain, disability, presenteeism and absenteeism, which reflected improved quality of life. Provider views on adherence appeared discordant with patient claims, and did not predict target achievement. Never smoking, lower body mass index, and lower prednisone dose (7.5 mg daily) were independently associated with a higher likelihood of achieving T2T therapeutic goals after 6 months.A combination of clinical characteristics and provider treatment decisions shapes the "profile" of a patient failing to achieve T2T goals. Low‑dose steroid equivalent, never smoking, and lower body mass index appear as individual characteristics independently associated with achieving LDA / remission at 3 and 6 months.

Idiopathic retroperitoneal fibrosis, or Ormond’s disease, is a rare syndrome which is in essence the deposition of pathological fibrous tissue in the retroperitoneal space. In most cases, it is initially asymptomatic, but as the disease progresses, clinical symptoms develop. The main complaint are abdominal pain and symptoms resulting from the involvement of ureters, arterial and venous vessels. Diagnosis is based on computed tomography or magnetic resonance imaging, demonstrating a fibrotic tissue mass accumulated in the retroperitoneal space. Due to the typically late presentation of clinical symptoms, a diagnosis is difficult and is often made after complications occur. In our article, we present the case of a 71-year-old patient in whom coexisting ankylosing spondylitis and retroperitoneal fibrosis were diagnosed 15 years after non-specific abdominal pain occurred.

The long-term mortality in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) is unexpectedly decreased or at least not increased regardless of several mortality risk factors that these diseases share with other chronic immune-mediated rheumatic diseases. The genetic and immunological profile of PMR/GCA patients is unique, therefore, the hypothesis that this profile provides some survival advantage to PMR/GCA patients should be considered. The longevity is a phenomenon that was demonstrated to be familial. The familial aggregation of longevity can be studied by analysis of life expectancy in family members. Here we test the hypothesis of the aggregation of an increased longevity in the families of PMR/GCA patients. We compared the age of death of 358 parents of 179 PMR and GCA patients with corresponding data retrieved from 506 parents of 253 randomly collected age and sex-matched controls. The number of nonagenarian (≥ 90-year -old) mothers of PMR/GCA patients was significantly higher (OR = 2.34, 95%CI 1.11–11.95, p p = 0.003). Our data suggest the familial aggregation of nonagenarians in PMR/GCA patients.

Introduction: Real-world data indicate disparities in biologic access across Europe. Objectives: To describe the national structure of PsA care in Poland, with a particular focus on the population of inadequate responders (IRs) and difficulties associated with biologic therapy access. Methods: A pool of rheumatologic and dermatologic care centers was created based on National Health Fund contract lists (n = 841), from which 29 rheumatologic and 10 dermatologic centers were sampled randomly and successfully met the inclusion criterium. Additionally, 33 tertiary care centers were recruited. For successful center recruitment, one provider had to recruit at least one patient that met the criteria for one of the four pre-defined clinical subgroups, in which all patients had to have active PsA and IR status to at least 2 conventional synthetic disease-modifying drugs (csDMARDs). Self-assessment questionnaires were distributed among physicians and their patients. Results: Barriers to biologic DMARD (bDMARD) treatment are complex and include stringency of reimbursement criteria, health care system, logistic/organizational, and personal choice factors. For patients who are currently bDMARD users, the median waiting time from the visit, at which the reimbursement procedure was initiated, to the first day of bDMARD admission was 9 weeks (range 2–212, 32% &lt, 4 weeks, 29% 5–12 weeks, 26% 13–28 weeks, 13% with &gt, 28 weeks delay). Out of all inadequate responder groups, bDMARD users are the only group with “good” therapeutic situation and satisfaction with therapy. Patient satisfaction with therapy is not always concordant with physician assessment of therapeutic status. Conclusions: Despite the fact that over a decade has passed since the introduction of biologic agents, in medium welfare countries such as Poland, considerable healthcare system barriers to biologic access are present. Out of different IR populations, patient satisfaction with treatment is often discordant with physician assessment of disease status.

The existence of polymyalgia rheumatica (PMR) with normal inflammatory indices at the time of diagnosis still represents a diagnostic conundrum. According to the literature, some patients with PMR following immune checkpoint inhibitory therapy had normal values of both erythrocyte sedimentation rate and C-reactive protein concentrations at the time of diagnosis. In this short communication we investigated the possibility that in some patients with PMR the main pathogenic mechanism is constituted by inhibition of some checkpoints, such as programmed death receptor-1, programmed death ligand 1, and “cytotoxic” lymphocyte antigen 4. In these patients, the pathogenetic mechanisms underlying PMR can act much more upstream than commonly suggested. Also, we addressed the question of whether these patients should be considered as affected by PMR-like syndromes or by PMR subset.

Introduction Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs, leading to their failure and disturbances in the morphology and function of blood vessels. The disease affects people in different ways, and identifying how the difficulties and limitations are related to quality of life may contribute to designing helpful interventions. The aim of this study was to identify factors associated with quality of life in people with SSc. Methods This was a cross-sectional study conducted in 11 rheumatic centres in Poland. Patients diagnosed with SSc were included. Quality of life was measured using the SSc Quality of Life Questionnaire (SScQoL). The following candidate factors were entered in preliminary multivariable analysis: age, place of residence, marital status, occupational status, disease type, disease duration, pain, fatigue, intestinal problems, breathing problems, Raynaud’s symptoms, finger ulcerations, disease severity, functional disability, anxiety and depression. Factors that achieved statistical significance at the 10% level were then entered into a final multivariable model. Factors achieving statistical significance at the 5% level in the final model were considered to be associated with quality of life in SSc. Results In total, 231 participants were included. Mean age (SD) was 55.82 (12.55) years, disease duration 8.39 (8.18) years and 198 (85.7%) were women. Factors associated with quality of life in SSc were functional disability (β = 2.854, p

Objective The aim of this study was to investigate long-term clinical outcomes of extended treatment with CT-P10, a rituximab biosimilar, compared with rituximab reference products sourced from the USA and the EU (US-RTX and EU-RTX) in rheumatoid arthritis (RA) for up to 48 weeks. Methods In this multinational, randomized, double-blind trial, adults with active RA received up to two courses of CT-P10, US-RTX, or EU-RTX alongside methotrexate. Efficacy endpoints included Disease Activity Score 28-joint count (DAS28) and American College of Rheumatology (ACR) response rates. Pharmacokinetics, pharmacodynamics, immunogenicity, and safety were also assessed. Results Of 372 patients randomized to the study drug, 330 (88.7%) completed the second treatment course. Mean change from baseline to week 48 in DAS28-C-reactive protein was comparable in the CT-P10 and combined rituximab (US-RTX and EU-RTX) groups (− 2.7 and − 2.6, respectively). ACR20, ACR50, and ACR70 response rates at week 48 indicated no differences between groups (80.6%, 55.4%, and 31.7% vs. 79.8%, 53.9%, and 33.7% in the CT-P10 and combined rituximab groups, respectively). Similar improvements in the Health Assessment Questionnaire Disability Index and all medical outcomes in the Short Form 36-Item Health Survey, including physical and mental health, were seen in all groups. At week 48, antidrug antibodies were detected in 4.9%, 9.4%, and 8.6% of patients in the CT-P10, US-RTX, and EU-RTX groups, respectively. CT-P10 and rituximab displayed similar pharmacokinetic, pharmacodynamic, and safety profiles. Conclusion CT-P10 was similar to EU-RTX and US-RTX in terms of efficacy, pharmacokinetics, pharmacodynamics, immunogenicity, and safety up to week 48. ClinicalTrials.gov identifier NCT02149121. Electronic supplementary material The online version of this article (10.1007/s40259-018-00331-4) contains supplementary material, which is available to authorized users.

AIM: To evaluate the incidence of increased intraocular pressure (IOP) and glaucomatous changes in systemic lupus erythematosus (SLE) patients in comparison with systemic steroids and immunosuppressive treatment. METHODS: Sixty-two women with SLE were divided into two groups: treated (n=47, 94 eyes) and not treated (n=15, 30 eyes) with systemic glucocorticosteroids (GC; GC-free). Twenty-one individuals in GC group were treated with immunosuppressive agents (immunomodulating and biologic). The visual acuity and IOP with ocular pulsatile amplitude (OPA) measurements, as well as scanning laser polarimetry (GDx) with nerve fiber index (NFI) measurement, spectral domain optical coherence tomography (SD-OCT) of the optic disk with retinal nerve fiber layer (RNFL) analysis and the macular region with ganglion cell analysis (GCA) were performed. RESULTS: Mean IOP values in group with combined GC and immunosuppressive therapy was 15.8±2.56 mm Hg and was significantly lower than in individuals with exclusive GC treatment (17.63±4.38 mm Hg, P=0.043). Contrary, no differences in mean IOP values between GC-free group and individuals treated with combined GC and immunosuppressive therapy were detected (P=0.563). Similarly, mean IOP in GC was 17.14±3.94 mm Hg and in GC-free patients was equal to 16.67±3.45 mm Hg (P=0.671). According to treatment regimen no statistical differences in optic disk SD-OCT for RNFL thickness, RNFL symmetry, cupping volume and the C/D ratio were observed. Similarly, no statistical differences for the mean and minimal ganglion cell layer (GCL) thickness measured in macular SD-OCT or NFI in GDx were detected. CONCLUSION: Combined immunosuppressive and systemic GC therapy in SLE patients may lower the risk of iatrogenic ocular hypertension. No relationship between treatment regimen and glaucomatous damage of optic nerve fibers in analyzed groups with SLE is detected.

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%-12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a constellation of dermatological and osteoarticular symptoms. The pathogenesis of SAPHO is unknown, but infectious, genetic, immunological and environmental factors may play a role. SAPHO is classified along two different spectrums: pustulo-psoriatic hyperostotic spondyloarthritis and chronic recurrent multifocal osteomyelitis. The typical skin lesions are palmoplantar pustulosis and acne. The sign of arthritis is involvement of the anterior chest wall, most often the sternoclavicular joints. There are no standard treatment recommendations, but nonsteroidal anti-inflammatory drugs, sulfasalazine, methotrexate, antibiotics and biological drugs can be considered.

Silent vasculitis may accompany polymyalgia rheumatica (PMR), resulting in complications such as vision deterioration. Reduced visual field is a possible manifestation. A 66-year-old man was diagnosed with PMR and treated accordingly (prednisone maximum 15 mg/day) at a tertiary center, with resolution of his symptoms. After 18 months, he suddenly experienced right eye vision loss. Visual field of his left eye was peripherally limited (Figure 1). Color Doppler ultrasound …

INTRODUCTION ANCA-associated vasculitides (AAV) is a group of rare disorders where inflammation and damage of the small blood vessels lead to dysfunction of the supplied organs. In severe flares of the disease patients may require intensive care unit (ICU) admission and treatment. The study aims to characterize Polish patients with AAV who were admitted to the ICU and compare them to the others. MATERIAL AND METHODS An observational, retrospective study based on the POLVAS - registry of Polish adult patients with AAV was carried out. Patients admitted to the ICU (ICU group) were identified and compared with the patients who did not require ICU admission (non-ICU group). Characteristics and comparison between groups were made using standard statistic descriptive methods. RESULTS 30 patients admitted to the ICU were identified among 573 cases included in the registry. All patients in the ICU group with available data were ANCA positive. The clinical manifestations related to the ICU admission were respiratory, renal and central nervous system involvement. The treatment regimen for remission induction was similar in both groups. Almost half of the patients in the ICU-group (48.3%) required dialysis, whereas in the non-ICU group it was 21.8% (P = 0.01). Infections were also more frequent in the ICU group (72.4% vs. 36.9% P < 0.001). The mortality rate among patients who needed ICU treatment was significantly higher when compared to the rest of the patients (53.6% vs. 7.8%; P < 0.001). CONCLUSIONS In the Polish AAV cohort one in twenty patients required ICU admission. This group was characterized by multiple organ involvement and high mortality.

Kazdego roku coraz wiecej osob z chorobami reumatycznymi jest kwalifikowanych do calkowitej alloplastyki stawu biodrowego i/lub kolanowego. Przygotowanie chorego do operacji jest trudne nie tylko dla ortopedy, ale moze budzic rowniez wątpliwości wśrod lekarzy innych specjalności. Mnogośc lekow stosowanych w reumatologii, ich dlugotrwale dzialanie immunosupresyjne moze istotnie wplywac na c zestośc powiklan w okresie okolooperacyjnym. Czestym i bardzo trudnym w leczeniu powiklaniem po endoprotezoplastyce stawow są zakazenia okoloprotezowe. Wlaściwe okolooperacyjne przygotowanie chorego pomaga zmniejszyc ryzyko infekcji. Kluczowe jest szybkie zdiagnozowanie problemu i wdrozenie wlaściwego leczenia do 3 tygodni od pierwotnego zabiegu operacyjnego. Opoźnione lub niewlaściwe leczenie zajmuje wiele tygodni i ma mniejsze szanse powodzenia. Forum Reumatol. 2018, tom 4, nr 3: 163–168

Evidence from over 10 years of clinical experience demonstrates that biosimilar medicines approved in the European Union can be used for all their registered indications as safely as their originators and with no negative impact on therapeutic efficacy. The debate on the use of biosimilars in rheumatology focuses specifically on the safety of switching between biosimilars and reference products. Studies conducted to date, including randomised double-blind and open-label extension trials, have not demonstrated any significant differences in therapeutic efficacy or safety between patients switched from one medicine to another and those who were continued on a single medicine. According to the latest recommendations for the use of biosimilars in rheumatic diseases, developed by an international task force in 2017, there is no clinical evidence that a single switch from an originator to a biosimilar medicine is associated with any significant risk for patient safety or reduction in therapeutic efficacy.

Tofacitinib is a newly approved small-molecule targeted synthetic disease-modifying antirheumatic drug. The drug was designed as a selective and specific inhibitor of pro-inflammatory receptor signalling. Tofacitinib inhibits the process of intracellular signalling from the receptor to the cellular nucleus and inhibits the inflammation process via a new pathway (inhibition of the Janus kinases), which is unavailable to biological medicines. Tofacitinib has been approved for use in the treatment of patients with moderate to severe active RA. The drug may be used in combination with methotrexate or another conventional synthetic disease-modifying antirheumatic drug or in monotherapy. The efficacy of tofacitinib has been confirmed in several clinical trials. The drug inhibits radiographic progression of the disease. The innovative mechanism of action of tofacitinib is a noteworthy feature because it offers hope of effective treatment for patients who fail to respond to other drugs. The presented article discusses the mechanism of action and the clinical application of tofacitinib. Tofacitinib represents a new group of disease-modifying antirheumatic drugs that can be placed on an equal footing with biological drugs already available.

Objectives: Report on one-year results from the Polish Spondyloarthritis Initiative registry (PolSPI), containing the cross-sectional analysis of clinical and imaging data as well as database methodology. Material and methods: The PolSPI registry includes patients with axial (axSpA) and peripheral (perSpA) spondyloarthritis according to ASAS classification criteria, and/or patients with ankylosing spondylitis according to modified New York criteria, psoriatic arthritis according to CASPAR criteria, arthropathy in inflammatory bowel disease, reactive arthritis, juvenile spondyloarthritis or undifferentiated spondyloarthritis. Epidemiologic data and history of signs, symptoms and treatment of spondyloarthritis are collected and assessment of disease activity is performed. Radiographic images of sacroiliac joint, cervical and lumbar spine, and results of bone densitometry are collected. Every 6 months blood samples for inflammatory markers, and for long-term storage are taken. Results: During a one-year period from September 2015 to August 2016, 63 patients were registered on an electronic database; 44 (69.8%) of patients were classified as axial spondyloarthritis (axSpA) and 19 (30.2%) as peripheral spondyloarthritis (perSpA) according to ASAS criteria. Statistically significant differences between axSpA and perSpA were discovered in the percentage of HLA-B27 antigen occurrence (92.6% and 50%, respectively), BASDAI (2.8% and 4.1%, respectively), DAS 28 (2.66% and 4.03%, respectively), percentage of peripheral arthritis (20% and 88.8%, respectively), enthesitis (26.7% and 70.6%, respectively), dactylitis (6.7% and 88.9%, respectively), as well as extra-articular symptoms: acute anterior uveitis (26.7% and 5.6% , respectively) and psoriasis (6.9% and 55.6%, respectively). Patients with axSpA had significantly higher mean grade of sacroiliac involvement according to New York criteria, higher mSASSS score, and lower T-score in femoral neck in bone densitometry. Conclusions: At the early stage of the disease patients with axSpA compared to those with perSpA, have more advanced structural damage of sacroiliac joints and spine, and lower bone mineral density in the femoral neck. In the upcoming years the PolSPI registry will prospectively follow-up patients with SpA, recording response to treatment and carrying out research on interaction of inflammation and bone remodelling.

The myxoma makes 80–90% of benign cardiac neoplasms. The first symptom in 50% of patients is an embolism resulting from relocation of tumor fragments or blood clots into the bloodstream. Elevated values of acute phase proteins in these patients are results of non-specific immune response to an antigen causing the disease. Interleukin 6 (IL-6) plays a role in this reaction, modifying inflammatory response by: influence on lymphocyte T differentiation, lymphocyte B to plasmocyte transformation, and stimulation of the liver to produce acute phase proteins. Elevated IL-6 is found in 80% of patients with diagnosed myxoma, which causes it to be an important marker in diagnostic and post-operational monitoring. The role in non-specific inflammatory response played by IL-6 in myxoma and autoimmune disorders was a cause of many diagnostic mistakes. Available literature does not suggest a coincidence of cardiac myxoma and systemic sclerosis. This is why we would like to present a case report, with special regard to correlation between IL-6 values and activity/stage of diagnosed disorders.

Polymyalgia rheumatica (PMR) is a unique disease of elderly people, traditionally diagnosed based on a clinical picture. A typical case is a combination of severe musculoskeletal symptoms and systemic inflammatory response with spectacular response to corticosteroids treatment. The severity of symptoms may be surprising in older patients where immunosenescence is normally expected. However, PMR may be diagnosed in haste if there is a temptation to use this diagnosis as a shortcut to achieve rapid therapeutic success. Overdiagnosis of PMR may cause more problems compared to underdiagnosis. The 2012 PMR criteria proposed by European League against Rheumatism/American College of Rheumatology aim to minimize the role of clinical intuition and build on more objective features. However, questions arise if this is possible in PMR. This has been discussed in this review.

Career situation of first and presenting author Assistant. Introduction Vascular abnormalities are common complications in rheumatic patients. Their background is multifactorial and still is a subject of the debate. Objectives This study was designed to evaluate the significance of selected noninvasive imaging indices, immunological and genetic biomarkers in diagnosis of vascular lesions in rheumatic diseases. Methods The study group included 288 patients with systemic connective tissue diseases (SCTD). The noninvasive evaluation of vascular lesions was made on the basis of carotid intima-media thickness (cIMT), ankle brachial index (ABI), high resistance index (HRI) and ulnar artery intraluminal diameter (UAID) measurements using HDI 3500 (ATL). We analyzed more than 100 variables: autoantibodies, inflammatory and angiogenic markers, genetic polymorphisms and classical risk factors for atherosclerosis. Statistical analysis was performed with STATA 11 including: chi2Yates, chi2Pearson and rank Spearman correlations tests, logistic regression analysis and multivariate stepwise analysis. Results Macroangiopathy was influenced by selected autoantibodies including antiphospholipid (OR=4.4; 95% CI:1.1–20.7) and anti-endothelial cell (OR=6.6; 95% CI:1.6–28.3) as well as inflammatory biomarkers (OR=3.6; 95% CI:1.1–11.8). The analysis of genetic polymorphisms showed especially an important impact of VEGF 2578 AA genotype on atherosclerosis development (OR=4.8; 95% CI:1.1–21.1). Angiogenic biomarkers were strongly associated with prothrombotic risk (OR=22.8; 95% CI:2.3–230.6). The analysis of relations between imaging indices and vascular manifestations revealed significant association of cIMT with cardiovascular (OR=52.9; 95% CI:7.0–1012.7) and cerebrovascular disease (OR=4.0; 95% CI:1.0–15.3). There was significant reverse correlation between ABI and peripheral vascular disease (R=−0.33; p=0.001). HRI values significantly correlated with thromboembolic disorders (R=−0.29; p=0.03). Finaly, UAID was notably related to microangiopathic complications (p Conclusions The protocol for vascular lesions diagnosis in SCTD should be based on the combination of imaging and laboratory biomarkers. Immunological and inflammatory factors are crucial in diagnostics of vascular involvement in rheumatic diseases. IMT and ABI showed a high prognostic value and can be used for the general cardiovascular risk stratification. Disclosure of Interest None declared.

Author(s): Deodhar, Atul; Gensler, Lianne S; Sieper, Joachim; Clark, Michael; Calderon, Cesar; Wang, Yuhua; Zhou, Yiying; Leu, Jocelyn H; Campbell, Kim; Sweet, Kristen; Harrison, Diane D; Hsia, Elizabeth C; van der Heijde, Desiree | Abstract: ObjectiveTo evaluate the efficacy and safety of ustekinumab in 3 randomized, placebo-controlled studies in patients with axial spondyloarthritis (SpA). Studies 1 and 2 included patients with radiographic axial SpA (anti-tumor necrosis factor [anti-TNF]-naive patients and patients with inadequate response or intolerance to anti-TNF, respectively); study 3 patients had nonradiographic axial SpA.MethodsIn all 3 studies, patients were randomly assigned (1:1:1) to receive subcutaneous ustekinumab at 45 mg or 90 mg or placebo up to 24 weeks, after which placebo-treated patients were rerandomized to receive ustekinumab at 45 mg or 90 mg. The primary end point in studies 1 and 2 was the proportion of patients who met the Assessment of SpondyloArthritis international Society criteria for 40% improvement in disease activity (achieved an ASAS40 response). The primary end point in study 3 was the proportion of patients who achieved an ASAS20 response. Other disease activity and safety measures were also evaluated. A week 24 analysis of study 1 was preplanned to determine continuation of studies 2 and 3.ResultsFor study 1, the primary and major secondary end points were not met, and the study was discontinued. As a result, studies 2 and 3 were prematurely discontinued before they were fully enrolled. For all 3 studies, neither ustekinumab dose group demonstrated clinically meaningful improvement over placebo on key efficacy end points. The proportion of patients experiencing adverse events in the ustekinumab groups was consistent with that in previous studies.ConclusionIn these 3 placebo-controlled trials, efficacy of ustekinumab in the treatment of axial SpA was not demonstrated. The safety profile was consistent with that of studies in other indications.

Purpose The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients’ cohort. Materials and methods Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities. Results There were 625 patients diagnosed with AAV included in this study: 417 cases of granulomatosis with polyangiitis (GPA; 66.7%), 106 cases of microscopic polyangiitis (MPA; 17.0%) and 102 cases of eosinophilic granulomatosis with polyangiitis (EGPA; 16.3%). The mean age at the date of diagnosis was 50.4 (±15.7) years and the median observational period amounted to 4.0 (2.0–8.0) years. Glucocorticosteroids (GCs) were the medicaments most frequently used for remission induction (593/622; 95.3%), followed by cyclophosphamide (487/622; 78.3%), rituximab (44/622; 7.1%), and methotrexate (39/622; 6.3%). GCs were also most frequently administered for maintenance therapy (499/592; 84.3%), followed by azathioprine (224/592; 37.8%), methotrexate (136/592; 23.0%) and mycophenolate mofetil (99/592; 16.7%). The median cumulative doses of cyclophosphamide and rituximab equalled 7.99 g (4.18–14.0) and 2000 mg (1500–2800), respectively. The most commonly observed adverse events included: infections - 214/551 cases (38.8%), which were associated with the time of observation (OR = 1.05; 95% CI 1.01–1.10), the use of GCs intravenous pulses (OR = 2.76; 95% CI 1.68–4.54) and need for haemodialysis (OR = 1.73; 95% CI 1.10–2.71). Conclusions Polish patients with AAV were predominantly treated according to appropriate guidelines. The most frequent adverse events were typical for usually administered immunosuppressive treatment.

Atherosclerosis (ATS) is a multifactorial disease which may be considered an immune/inflammatory response of intima to tissue damage. Systemic autoimmune rheumatic diseases (SARDs) are a heterogeneous group of disorders characterized by an immune response against self-antigens based on the interaction between genetic predisposition, dysregulation of the immune system, and environmental factors. Various SARDs are associated with accelerated and often premature ATS. Traditional risk factors alone fail to fully account for this phenomenon; thus it has been attributed to complex interactions between traditional risk factors and factors associated with the disease process and its treatment. As cardiovascular disease (CVD) still remains the leading cause of morbidity and mortality in SARDs, there is an immense need for an improved CVD prevention in this patients group. Noninvasive imaging assessment of vascular pathophysiology as well as selected biomarkers can help to improve risk assessment.

Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3–12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06–9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05–6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3–25.5), skin involvement (OR = 4.4; 95% CI = 1.02– 19.6), and age > 65 (OR = 6.3; 95% CI = 1.18–33.7). Conclusions In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.